Hodgkin's disease variant of Richter's syndrome: complete remission of the both malignancies after 14 years.

Richter's syndrome is defined by the occurrence of high-grade malignant lymphoma in the course of chronic lymphocytic leukemia (CLL). The prognosis is poor with a life expectancy of a few months. We report on the case of a 68-year old male who developed Hodgkin's disease 6 years after the diagnosis of stage A CLL was made. The patient received 3 cycles of combination chemotherapy according to the ABVD regimen, followed by radiotherapy. This treatment resulted in complete apparent remission of the two diseases, which was persistent after a follow-up of 14 years. The Hodgkin's disease variant of Richter's syndrome is exceptional with about 20 reported cases to date. Recent research indicates that lymphoma cells derive from leukemia. The prognosis appears far better than that for the classical non-Hodgkin Richter's syndrome.

Stage I-IIE primary non-Hodgkin's lymphoma of the testis: results of a prospective trial by the GOELAMS Study Group.

Sixteen patients with aggressive primary testicular involvement were analyzed separately from a prospective multicenter series of 494 patients with stage I/II aggressive nonlymphoblastic lymphoma. The treatment strategy included 3 cycles of anthracycline-based chemotherapy followed by regional radiation therapy on inguinal, iliac, and para-aortic lymph nodes and central nervous system (CNS) prophylaxis by intrathecal chemotherapy and brain irradiation. Chemotherapy was stratified by age group. Patients aged 18-60 years received the Groupe Ouest Est d'Etude des Leucemies Aigues et Maladies du Sang (GOELAMS) 02 protocol: 3 monthly cycles of VCAP (vindesine 3 mg/m2 day 1, doxorubicin 80 mg/m2 day 2, cyclophosphamide 1500 mg/m2 day 2, and prednisone 80 mg/m2 days 1-5). Patients aged 61-75 years received the GOELAMS 03 protocol: 3 monthly cycles of VECP-Bleo (vindesine 3 mg/m2 day 1, epirubicin 60 mg/m2 day 1, cyclophosphamide 750 mg/m2 day 1, prednisone 50 mg/m2 days 1-7, and bleomycin 10 mg/m2 days 1 and 5). Sixteen patients had testicular involvement (3.3%). Median age was 62 years (range, 29-73 years). The histological subtypes were diffuse large-cell lymphoma in all cases. Ann Arbor stage was IEA in 11 patients, IEB in 3 patients, and IIEA in 2 patients. All patients achieved a complete response. One patient died from septic shock during the last course of chemotherapy. After a median follow-up period of 73.5 months, the probability of disease-free survival (DFS) and overall survival (OS) were 70% and 65%, respectively for all patients. Disease-free survival and OS were 66% and 83% in patients = 60 years of age, and 74% and 56% in patients > 60 years of age. Relapse occurred in extranodal sites in 4 cases and in abdominal lymph nodes in the last case. Relapse in the CNS occurred in only 1 patient and in the contralateral testis in 1 patient. We found no correlation between OS, DFS and extent of testicular involvement, Ann Arbor stage, International Prognostic Index score, or lactate dehydrogenase level. This is the first report of a prospective study in which treatment of testicular non-Hodgkin's lymphoma was precisely defined at diagnosis. Compared to other series, a combination of orchiectomy with 3 cycles of CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone)-derived chemotherapy, regional radiation therapy, and CNS prophylaxis seems to improve prognosis. The improvement in prognosis seemed to be due in part to irradiation, including the pelvic and lomboaortic lymphatic areas, and in part to CNS prophylaxis.

Neurosarcoidosis Associated with Hodgkin's Disease; Malignancy.

Sarcoidosis is a granulomatous disease of undetermined origin. The association of sarcoidosis with malignant lymphoma is a rare but possibly nonfortuitous event. The authors report on the case of a 26-year-old male patient presenting with lymphnode sarcoidosis which evolved into neurosarcoidosis. The evolution was responded to corticosteroid therapy. Thirty-three months after the diagnosis of neurosarcoidosis, the patient presented with cervical Hodgkin's disease which was successfully treated by chemotherapy and radiotherapy. The pathophysiological mechanisms and possible associations are briefly discussed.